Recent Advances 32 y/o female referred to our clinic for an - PowerPoint PPT Presentation

2/16/2018 History Recent Advances • 32 y/o female referred to our clinic for an increase in headaches for the past three months in Neurology Case Presentation • History of headaches since childhood – provoked by hunger or lack of sleep Rebecca L. Michael, MD • In adolescence, frequency increased with onset February 16, 2017 of menses and then again with onset of using OCPs History continued History continued • She endorsed a preceding visual aura • Location was unilateral frontotemporal region, – alternating wavy lines and black spots alternated sides equally – evolved into her entire visual field over 30 • Quality is described as pressure and at times minutes throbbing – occurred 75% of the time • Associated with nausea, photophobia, phonophobia and osmophobia. • She stopped OCP’s at age 28 with improvement in HA frequency to only 2-3 days/month 1

2/16/2018 History continued History continued • Past Medical History • Prior three months, headaches increased – menorrhagia s/p thermal ablation and eventual • 18 headache days per month and 8 days of “headache hysterectomy hangover” – hyperthyroidism s/p thyroidectomy • Quality of pain is similar – anxiety • Worse in the morning – obesity • Worse with valsava and bending over • Review of Systems • Visual ‘aura’ now however is “blacked out” central vision – snoring – insomnia • Only clear precipitant is stress with her upcoming wedding Question 1 History continued Based on the history, what is the most likely • Headaches are minimally responsive to tylenol diagnosis? and advil A. Chronic Migraine 44% • Rizatriptan and intranasal sumatriptan caused B. Sleep Apnoea Headache 37% side effects C. Idiopathic Intracranial • Has never been on a preventive medication Hypertension, IIH 11% for migraine D. Chronic Tension Type Headache 5% 3% E. Cerebral Venous Sinus Thrombosis e e n h . . . i c . . . . a a . e . T r H g d H s a l M i a u e e n H n i p S i c a y i a r T n s o e c n u o a o r r o h n t n p s i C n e A I n V e p c i T a l e h t c r e a i b l n S p o e o r r e i h d C I C 2

2/16/2018 Chronification of Migraine Neurological Exam BP: 115/73 HR: 78 BMI 32.94 • usually develops from episodic migraine • CN intact, visual acuity normal (annual conversion rate of about 3%) • Visual fields: enlarged blind spot bilaterally • optic discs blurred bilaterally • Risk factors – obscuration of all borders • remainder of neurological exam normal – Non-modifiable • age, female sex, low education status – Modifiable • MOH, ineffective acute treatment, obesity, depression and stressful life events May A, Schulte LH. Chronic migraine: risk factors, mechanisms and treatment Nature Reviews Neurology 12, 455–464 (2016) Question 2 IIHTT The most common headache phenotype • Largest randomized controlled trial on IIH ever (according to ICHD-3) described in patients with conducted IIH in the Idiopathic Intracranial Hypertension • 38 neuroophthalmology centers in US Treatment Trial, IHTT, is which of the following • 165 subjects 49% – Bilateral papilledema, mild visual field loss, lumbar A. Migraine puncture with elevated OP B. Probable migraine 27% • acetazolamide w/ diet (70) vs. placebo w/ diet (69) C. Tension-type headache 14% • Primary outcome 9% D. Probable tension-type headache – Perimetric mean deviation (PMD) at 7 months • Numerous secondary outcomes e e e n h n a i i c . . r a a . g r d e g M i i a p m e y h t e n - l e b p o a i y s b t n o - n e r o t P s i e l n b e a T b o r P 3

2/16/2018 Headache Characteristics at Baseline • HA present in 84% at baseline • Locations of headache Frontal 68% Global 36% Ocular 47% Posterior 39% Nuchal 47% Unilateral 30% • Characteristic of pain pressure like 47% throbbing 42% Friedman DI et al. Headache 2017; 57, 1195-1205 Headache Characteristics at Baseline Headache Characteristics at Baseline • “Migraine” associated symptoms common • Headache phenotype (ICHD-3 beta) photophobia (70%), phonophobia (52%), nausea (47%), Migraine 52% Probable migraine 16% vomiting (17%), worsened with physical activity (50%) Tension-type 22% Probable tension-type 4% Not classifiable 7% • IIH-related symptoms • 50% of migraine or probable migraine subjects (n=94) had a • Constant visual loss (34%) history of migraine • Transient visual obscurations (68%) • Diplopia (22%) • 29% of the other headache types (n=45) had a history of • Dizziness (53%) migraine • Headache with none of the above (14%) Friedman DI et al. Headache 2017; 57, 1195-1205 Friedman DI et al. Headache 2017; 57, 1195-1205 4

2/16/2018 Back to our case… MRI brain wwo contrast • 32 y/o female w/ increase in headache frequency and papilledema • Referred for urgent evaluation/imaging • Ophthalmology exam MR venogram • Lumbar Puncture – initial opening pressure was 25cm 5

2/16/2018 Criteria for IIH Diagnosis Criteria for IIH Diagnosis Initially defined by Dandy and modified by Diagnosis without papilledema Friedman et. al – if B-E are satisfied in addition to a bilateral or unilateral abducens palsy 1. Required for the diagnosis – Diagnosis can be suggested if B-E and three of four A. Papilledema neuroimaging are satisfied B. Normal neurological exam except for cranial nerve abnormalities i. Empty sella C. Neuroimaging (MRI brain wwo contrast for typical ii. Flattening of posterior aspect of the globe patients and MRV for others) iii. Distention of perioptic subarachnoid space with or D. Normal CSF composition without a tortuous optic nerve E. Elevated lumbar puncture OP (≥25 mm in adults, ≥28 iv. Transverse venous sinus stenosis mm in children) Friedman DI et al. Neurology 2013 Friedman DI et al. Neurology 2013 Neuroimaging Findings Back to our case… • Diagnosis of Idiopathic Intracranial Hypertension was made • Initiated on acetazolamide 500mg BID • Was seen by nutritionist • Referred for sleep medicine evaluation 6

2/16/2018 Follow Up Optical Coherence Tomography, OCT • Retinal Nerve Fiber Layer (RNFL) Thickness • Lost 12 lbs • Headache reduced to prior frequency of 2-3 days/month • Denied further TVO’s • Repeat ophthalmology exam – Papilledema no longer present – Optical Coherence Tomography, OCT, normal • Repeat LP with opening pressure 30cm – CSF normal Mollan et al. J Neurol Neurosurg Psychiatry, 2016 Question 3 Management OCT, optical coherence tomography, is unreliable • Instructed on slow taper diamox in which of the following scenarios? • Naratriptan given for acute treatment 39% A. significant disc edema • Encouraged continued weight loss 32% B. crowded disc • Called three weeks later with increase in C. pseudopapilledema 16% headache frequency and severity 12% D. answer choices A and B • Initiated on topamax 25  100mg 1% E. answer choices A, B and C • Currently doing well a c a B C m s m d i d d e n e n d d d a a e e e A B d l c l s w i s A , i p e d o a c s t r p i e c o o n c a d h i u c o c h i e r f e c n i s p w r g e s s i n w a s n a 7

2/16/2018 Management Management • In the IHTT, acetazolamide + weight loss program improved visual outcomes – more so than weight loss program alone • acetazolamide is safe up to 4 g/day • alternatives – Topiramate – Zonisamide – Furosemide ? – Indomethacin, Iron Friedman DI et al. Headache 2017; 57, 1195-1205 Friedman DI et al. Headache 2017; 57, 1195-1205 Conclusion Thank You • UCSF Headache Center Referrals: • IIH is a condition important to recognize because it may – Intractable migraine, cluster headaches, post-traumatic lead to progressive loss of vision over time, which may be headaches and other unusual or difficult headache permanent disorders • Diagnosis can be challenging – Outpatient treatment – Overlap with migraine – Nerve blocks – May present with papilledema or without – Neurostimulation – Telemedicine • OCT may help aid in the diagnosis of mild cases or in the monitoring of response to treatment – Research – Inpatient treatment • Additional headache treatments should be undertaken 8

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